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Eur J Pediatr. 2002 Oct;161(10):524-7. Epub 2002 Aug 22.

Severe transient myocardial ischaemia caused by hypertrophic cardiomyopathy in a patient with congenital disorder of glycosylation type Ia.

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1
Klinik und Poliklinik für Kinderheilkunde, Albert-Schweitzer-Strasse 33, 48149 Münster, Germany. marquat@uni-muenster.de

Abstract

Severely affected children with congenital disorder of glycosylation type Ia (CDG-Ia; MIM 212065) may develop hypertrophic cardiomyopathy. In this report we describe the near-death of a 10-month-old girl with CDG-Ia due to acute left-ventricular outlet obstruction caused by hypertrophic cardiomyopathy and acute dehydration. The girl had multi-organ failure and signs of severe myocardial damage mimicking myocardial infarction.

CONCLUSION:

hypertrophic cardiomyopathy contributes to the high mortality of young children with congenital disorder of glycosylation type Ia. Even if cardiomyopathy in this disease is non-obstructive, acute fluid-loss might cause left ventricular outflow tract obstruction and life-threatening myocardial ischaemia. Patients with congenital disorder of glycosylation type Ia are at risk for cardiac complications and should be monitored regularly by echocardiography.

PMID:
12297897
DOI:
10.1007/s00431-002-1029-2
[Indexed for MEDLINE]
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